BACKGROUND: Brain stem dermoid cysts are very rare lesions, and in most, the outcome has been very poor. Because of the dangers of dissecting the cyst wall away from the brain stem parenchyma, some authors have advocated not to attempt a radical resection. METHODS: We describe a child in whom the brain stem dermoid cyst recurred rapidly after a conservative approach. We therefore attempted a radical removal. RESULTS: During surgery, the almost complete resection of the cyst wall was not very difficult, leading to an apparent cure after 4 years. CONCLUSION: In exceptional cases, it may be possible to remove a brain stem dermoid cyst without prohibitive morbidity and with long-term cure.